Search

Congenital lobar emphysema is often associated with CHD in early infancy; however, the surgical strategy for this condition is still controversial. We report three successful cases of emphysematous lung lobectomy before the surgical repair of associated CHD. Aggressive lobectomy preceding cardiac interventions is advised when the management of congenital lobar emphysema is difficult.

We present a 31 gestational weeks’ premature baby whose fetal echocardiogram showed ectopia cordis, single ventricle and severe pulmonary stenosis. At 31 gestational weeks, an emergency caesarean section was performed, and his birth weight was 1756 g, SpO2 was 80% on 100% O2. Epicardial echocardiogram showed double inlet right ventricle, severe valvular pulmonary stenosis and no ductus arteriosus. The risk of surgery was very high, so we decided to perform balloon valvuloplasty by direct puncture of the heart. We punctured the apex of the ventricle using a 16-gauge needle under echo guidance, advanced the guidewire to the pulmonary artery and performed balloon valvuloplasty. Soon after the procedure, the cyanosis improved dramatically. This is the first report of a transcatheter procedure performed by direct puncture of the heart for ectopia cordis with complex congenital heart disease.

Objective This study aims to investigate the changes in renal function and levels of urinary biomarkers before and after cardiac angiography in children with congenital heart disease (CHD). Setting Children with CHD are at a risk for kidney injury during contrast exposure in cardiac angiography. Outcome measures We measured urinary protein, albumin, N-acetyl-β-D-glucosaminidase (NAG), β2-microglobulin (BMG), and liver-type fatty acid-binding protein (L-FABP) levels, as well as serum creatinine and cystatin C levels, before and after cardiac angiography in 33 children with CHD. Results No significant decrease was noted in either the creatinine-based or cystatin C-based estimated glomerular filtration rate at 24 hours after angiography compared with that before angiography. Urinary protein, NAG, BMG, and L-FABP levels were significantly increased at 24 hours after angiography, all of which returned to baseline levels at more than 7 days after angiography. An increase in urinary level of protein, albumin, NAG, or BMG was mostly associated with increased urinary L-FABP level. An increase in both urinary BMG and L-FABP, but not that in urinary L-FABP alone, was associated with increased levels of urinary protein and NAG, as well as the greater dose of contrast media. Conclusions Transient increases of kidney injury biomarkers following cardiac angiography are not necessarily associated with the impairment of renal function in a short time period; however, the increase in urinary protein, albumin, NAG, or BMG level may indicate greater stresses to the kidneys than the increase in urinary L-FABP alone in children with CHD.

症例は51歳女性. 15年来の糖尿病歴があり, 1995年7月, 糖尿病性腎症により血液透析導入. 当初より上腸間膜動脈血栓症に対する空腸切除術後のためと考えられる断続的な下痢症を認め, インスリンによる血糖コントロールは不良であった.1996年8月10日頃から徐々に全身倦怠感が出現. その後, 歩行時のふらつきと軽度の構語障害が出現したため, 頭部CTを施行するも特に病変を認めなかった. 8月20日にはNa 125mEq/lと低Na血症を認めたため, 食塩の摂取を促した. 8月21日に頭部MRIを施行したところ, 橋中央部に低信号域を認めた. 他の臨床所見と併せてcentral pontine myelinolysis (CPM) と診断した. 8月24日入院. 少量のNa補充と止痢剤の強化, および除水と厳密な血糖コントロールにより緩徐なNa補正を行い, 著明な神経学的改善を認めた.低Na血症の主な原因として, 断続的な下痢症によるNaの喪失や食事摂取量低下によるNa不足, および不適切な基準体重設定や高血糖に起因した高浸透圧血症による水過剰などが考えられた. また通常組成 (Na; 140mEq/l) の透析液による予期せぬ急速なNa補正がCPMを発症, あるいは増長した可能性が考えられた. 透析患者では電解質異常が起こりやすく, さらに透析療法自体が急速な電解質補正になりうるため, 臨床的にCPMが疑われた場合は迅速な診断かつ慎重な対処が必要であると考えられた.

The aim of this study was to examine whether the frequent short-time hemodiafiltration (FSHDF) could remove beta2-microglobulin (beta2m), an amyloidogenic factor, more effectively compared with the conventional hemodiafiltration (CHDF). A uremic patient underwent FSHDF (each 90 min, 6 times/week) for 6 weeks after CHDF (each 4 h, 3 times/week) for 6 months. Pretherapeutic plasma concentrations of beta2m, small molecules, electrolytes and bicarbonate were measured every Monday for 4 weeks during CHDF and for 6 weeks during FSHDF. The body weight gain during these weeks was also determined. The time average concentrations in beta2m and blood urea nitrogen (TACbeta2m and TACBUN) were evaluated based on their concentrations in pre-, post- and intertherapeutic plasmas frequently collected for 1 week each during CHDF and FSHDF. The quantities of total protein, albumin and beta2m in the removed fluids for the same week were determined. The pretherapeutic concentration in beta2m was significantly lower (18.7 +/- 1.3 mg/l) during FSHDF than CHDF (32.4 +/- 2.8 mg/l). The TACbeta2m was decreased during FSHDF (from 23.5 to 13.7 mg/l), while the TACBUN did not change. The pretherapeutic bicarbonate concentration was higher and the gain of body weight per week was lower during FSHDF although the other parameters were not different. It is concluded that FSHDF therapy is more effective to decrease beta2m concentration and correct acidosis together with an adequate urea removal and clinical safety.